Provide a holistic care plan of wounds on patients with Epidermolysis Bullosa

Introduction: Epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of severe inherited blistering diseases that affects 500,000 individuals worldwide. Clinically, the common factor in all types of EB is the tendency for skin and mucous membranes to blister or shear away in response to minimal everyday friction and trauma and show involvement of skin and mucosa membrane and other organs in some subtypes.Abstracts: The severity of EB varies from simple blistering affecting the hands and feet, particularly in warm weather, to death in early infancy from the devastating combination of laryngeal disease and failure to thrive. Those with DEB develop Micro-stomia and esophageal strictures as a result of contractures and scarring. People with more severe forms of EB can experience recurrent blistering and skin loss. Wound care has been highlighted as a priority and fundamental issue for patients, care givers, and health care professionals. (Davila-Seijo, 2013(In EB specifically, intact blisters and open wounds occur at various stages of healing and can be located anywhere on the body, but tend to be more pronounced on trauma-prone areas such as knees, feet, hands, and elbows, where the skin overlies bony prominences. Wounds may heal with scarring leading to contractures, most notable on the hands and feet, although large joints may also be affected. There is also a tendency to develop chronic wounds resulting from the underlying gene defect, compromised nutrition, chronic anemia and repeated infection, together with constant trauma. There is also a greatly increased risk of aggressive squamous cell carcinoma in those with severe forms of EB (Mellerio, Robertson et al, 2016.(Thankfully, there are now a variety of products that are suitable for wound care for EB. In this area a lot has happened in recent years. However, it is still not easy to find the right material for the patient. What is good and suitable for one person, may appear unsuitable for the next. The assessment of the individual products for the person themselves, or in babies and young children, are always done by the parents or caregivers. These various materials you should use one at a time, and once you have found one that seems right for your needs and one you can work with: keep it. It makes little sense to change the dressing type repeatedly to try new ones .Deciding which wound care materials should be used has been shown by experience and they cannot always be met entirely free from external circumstances. The availability of the products locally as well as the financial aspects must also be considered. There are different products in different countries, not all are available in all places. However, plus standard wound care, drug therapy, cellular and acellular matrix products, Cell therapy-fibroblasts, mesenchymal stromal cells (MSCs), Bone marrow Transplantation, protein therapy, gene therapy and Fractionated CO2 laser for EB wounds are modalities which use around world in everyday practice of health care professionals. Conclusion: However, Based on evidences for the use of specific wound management strategies, International consensus it would be chosen best practice guidelines of skin and wound care in Epidermolysis Bullosa.

کلیدواژه ها:

Wound care materials ، Epidermolysis Bullosa ، Holistic care plan ، Wound care modalities